Bleeding Disorders

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Common Disorders of Hemostasis: Primary Hemostasis/Inherited, Primary Hemostasis/Acquired, Secondary Hemostasis/Inherited, Secondary Hemostasis/Acquired

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1. Primary Hemostasis/Inherited: vWD
2. Primary Hemostasis/Acquired: Drugs, thrombocytopenia, uremia
3. Secondary Hemostasis/Inherited: Hemophilia (XIII or XI deficiency), Factor XI deficiency
4. Secondary Hemostasis/Acquired: Drugs, Liver disease, DIC, Vit K deficiency

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What does Von Willebrand Factor Do?

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1. Primary Hemostasis: Helps with platelet adhesion and aggregation
2. Secondary Hemostasis: chaperone for Factor XIII (crosslinks fibrin)

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Von Willibrand Disease: Clinical Presentation, Investigations, Treatment

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1. Clinical Presentation: variable bleeding/vWF levels, easy bruising, bleeding with major surgery, mucocutaneous bleeds (nose, gums, uterine), F>M
2. Investigations: PTT long/normal, vWF antigen decreased, vWF ristocetin factor maybe decreased, Factor VIII level maybe decreased
3. Treatment: DDAVP (mild), vWF/FVIII concentrates, anti-fibrinolytic agents

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Clinical Presentation, Investigations, Treatment

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1. Clinical Presentation:
2. Investigations
3. Treatment

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Clinical Presentation, Investigations, Treatment

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1. Clinical Presentation:
2. Investigations
3. Treatment

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Hemophilia A/Factor VIII deficiency: Clinical Presentation, Investigations, Treatment

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1.Clinical Presentation: X-linked recessive, deep bleeds (joints, muscle hematomas), GI/GU bleeds, severe surgical/trauma bleeds, positive family history
2.Investigations: PTT prolonged, PT/INR normal, decreased FVIII clotting activity and this correlates with severity of bleeding
3.Treatment: DDAVP (mild-moderate), FVIII concentrates, prophylactic replacement therapy (joint/muscle bleeds), Genetic counselling (female carrier detection, prenatal diagnosis)

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Hemophilia B/Factor IX deficiency: Clinical Presentation, Investigations, Treatment

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Clinical Presentation: X-linked recessive, deep bleeds (joints, muscle hematomas), GI/GU bleeds, severe surgical/trauma bleeds, positive family history
2.Investigations: PTT prolonged, PT/INR normal, FIX decreased activity
3. Treatment: Factor IX concentrates

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Factor XI Deficiency: Clinical Presentation, Investigations, Treatment

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Clinical Presentation: Autosomal Recessive, Ashkenazi Jews, bleeds with trauma/surgery, family history of bleeds
Investigations: PTT very long, PT/INR normal, FXI decreased activity (level DOES NOT correlate with bleeding)
3. Treatment: FFP, Factor XI concentrates

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Liver Disease Hemostasis: Clinical Presentation, Pathogenesis, Investigations, Treatment

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1. Clinical Presentation: esophageal/GI varices, gastritis, ulcers, bleeding with trauma/surgery
2. Pathogenesis: Decreased synthesis clotting factors (except VIII), aberrant synthesis of factors (defective clot), decreased clearance of activated factors, accelerated consumption of factors (DIC), Impaired fibrin formation, Thrombocytopenia/defective platelets
3. Investigations: Thrombocytopenia, Increased PT/PTT, Decreased Factors (except VIII), decreased/abnormal fibrinogen, increased FDPs/Ddimers (DIC)
4. Treatment: treat liver disease, vitamin K, replacement therapy (plasma, platelets, cryoprecipitate), DDAVP or anti-fibrinolytic agents (temporary)

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DIC: Clinical Presentation, Pathogenesis, Investigations, Treatment

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Clinical Presentation: systemic bleeding and/or clotting
Pathogenesis: Ongoing/uncontrolled generation of thrombin and plasmin due to excessive TF release (sepsis, trauma, cancer, obstetrical, vascular disorder, immunological disorder, toxins/drugs, liver disease) ->Intravascular fibrin formation, consumption of clotting factors, activation of fibrinolysis, generation of coagulation by-products (FDPs, D-dimers)
Investigations: Fragmented RBCs, decreased platelets/fibrinogen, prolonged PT/PTT, increased FDPs, D-dimers
Treatment: treat underlying cause (most impt), protein C+antithrombin concentrates (early DIC)

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Vitamin K deficiency: Clinical Presentation, Pathophsiology, Cause, Investigations, Treatment

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1. Clinical Presentation: Rising INR
2. Pathophysiology: Vit K activates II,VII,IX,X,protein C, protein S and will result in bleeding if active factors <10-15%
3. Causes: Warfarin, nutritional deficiency, antibiotics, biliary obstruction/malabsorption, hemorrhagic disease of the newborn
4. Investigations: Prolonged PT/PTT, Reduced II,VII,IX,X,protein C,protein S
5. Treatment: oral or IV vit K

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Differential Diagnosis of prolonged PT/INR and Normal PTT?

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1. Inherited: FVII deficiency
2. Acquired: Vit K deficiency, warfarin, liver disease

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Differential Diagnosis for Prolonged PTT and normal PT/INR?

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1. Inherited: Deficiency of VIII (hemophilia A),IX(hemophilia B),XI,XII, vWD
2. Acquired: Heparin, inhibitors (APLA)

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Differential Diagnosis for Prolonged PT/INR and PTT?

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Decreased/defective fibrinogen
Severe Vit K deficiency
Excessive warfarin/heparin
DIC
Severe liver disease